TY - JOUR T1 - Disease progression in IPF assessed using pulmonary function tests and functional respiratory imaging (FRI) – A pilot study JF - European Respiratory Journal JO - Eur Respir J VL - 44 IS - Suppl 58 SP - P654 AU - Jan De Backer AU - Wim Vos AU - Gerald Smaldone AU - Shaji Skaria AU - Rany Condos Y1 - 2014/09/01 UR - http://erj.ersjournals.com/content/44/Suppl_58/P654.abstract N2 - RationaleDisease progression in IPF is correlated with reduced lobar volume (iLobe), enlarged airway volume (iVaw) and reduced airway impedence (iRaw).Methods5 IPF patients were followed for 6 months to assess changes in airway structure and function. At baseline and after 6m FVC, DLCO and 3He-based FRC/TLC were measured. Also, HRCT-based functional respiratory imaging (FRI) was performed. FRI provides information on iLobe, iVaw and iRaw.ResultsIn terms of FVC, 1pt declined (-6%), 1pt improved (+6%) and 3pts remained stable (-1%,0%,1%). The pt that declined in FVC also experienced: DLCO -21%, FRC -12.67%, TLC -7.47%. iVaw stayed constant, iRaw decreased by 6.7%. However when iVaw/iRaw are corrected for lung volume (siVaw = iVaw/iLobe, siRaw = iRaw*iLobe), then siVaw increased by 23.99% and siRaw reduced by 30.90%. A negative but not significant correlation was found between changes in FVC and iVaw (R=-0.6, p=0.28). However, significant correlations were found between changes in FVC and siVaw (R=-1, p=0); changes in iLobe and siVaw (in the upper lobes R=-0.71, p=0.00283); and changes in siVaw and lobar ventilation (R=0.42, p=0.035).ConclusionsBy PFT criteria, IPF only progressed in one patient, but the study suggests that IPF progression is associated with a reduction in iLobe, an increase siVaw and a reduction in siRaw. Further studies need to confirm these initial observations. ER -